High prevalence of obstructive sleep apnea in Marfan's syndrome

Objective To review the current evidence about the prevalence of obstructive sleep apnea in patients with Marfan's syndrome, and discuss some proposed potential mechanisms for this relationship. Data sources The data in this review were mainly from Medline and PubMed articles published in English from 1990 to 2013. The search term was “Marfan's syndrome and sleep apnea”. Study selection Clinical evidence about the epidemiology of obstructive sleep apnea in patients with Marfan's syndrome; the mechanism that causes obstructive sleep apnea; interventional therapy for patients with Marfan's syndrome, and coexisting obstructive sleep apnea. Results A high prevalence of obstructive sleep apnea exists in patients with Marfan's syndrome. The potential reasons are craniofacial abnormalities and lax upper airway muscles, which lead to high nasal airway resistance and upper airway collapse. Obstructive sleep apnea mechanically deteriorates aortic dilatation and accelerates progression of aortic aneurysms. The condition is reversible and rapid maxillary expansion and adequate continuous positive airway pressure therapy are possible effective therapies to delay the expansion of aortic diameter in patients with Marfan's syndrome. Conclusions Obstructive sleep apnea is strongly associated with Marfan's syndrome. Craniofacial abnormalities and lax upper airway are the main mechanisms. Untreated obstructive sleep apnea accelerates progression of aortic dissection and rupture. Effective therapies for obstructive sleep apnea could postpone the aortic dilatation in patients with Marfan's syndrome.